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DOI 10.1002/ajmg.a.63961

Медицинская библиотека · American journal of medical genetics. Part A · 2024

Community-Sourced Reporting of Mortalities in Angelman Syndrome (1979-2022).

Gomes Adriana T, Moore Amanda, Cross Meagan, Hardesty Cristy, David Kelly, Sampedro Maria Galan, Dube Sophie, Weil-Chalker Sharon, Montepagano Adela Gentil, Christel Ursula, Martin Rachel, Wheeler Anne, Tan Wen-Hann, Bird Lynne M, Bichell Terry Jo

СтатьяПоддержка NIH: внешняя программаПеревод в очереди

Аннотация

Angelman syndrome (AS) is a severe genetic neurodevelopmental disorder with an estimated prevalence of 1:20,000. Life expectancy appears to be normal, however, data regarding lifespan in AS are scarce. Until 2018, there was no unique diagnosis code for AS, thus true incidence, prevalence, mortality and morbidity rates are unknown. A social media effort was launched by caregivers of people with AS to gather community-sourced data to understand AS mortality risks. Information on 220 deaths was verified with obituaries and public postings. Respiratory illness was the primary cause of death among people with AS overall, followed by accidents and seizures. Surprisingly, sudden unexpected death in sleep (SUDS) was the fourth most common cause, which has not been reported previously. Approximately 91% of people with AS have epilepsy, thus some SUDS cases may represent sudden unexpected deaths in epilepsy (SUDEP). Causes of death vary by age, and differ from the general population. Though there are obvious limitations to data collected through social media, grass roots science is a starting point to amass preliminary data and inform future epidemiological research on AS.

Для цитирования: Gomes Adriana T, Moore Amanda, Cross Meagan, Hardesty Cristy, David Kelly, Sampedro Maria Galan, Dube Sophie, Weil-Chalker Sharon, Montepagano Adela Gentil, Christel Ursula, Martin Rachel, Wheeler Anne, Tan Wen-Hann, Bird Lynne M, Bichell Terry Jo. Community-Sourced Reporting of Mortalities in Angelman Syndrome (1979-2022).. American journal of medical genetics. Part A. 2024. DOI: 10.1002/ajmg.a.63961.

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